DOI: 10.1073/pnas.0503460102. RPE65 is the isomerohydrolase in the retinoid visual cycle

RPE65 is the isomerohydrolase in the retinoid visual cycle

10.1073/pnas.0503460102

Crossref journal-article

Proceedings of the National Academy of Sciences

Proceedings of the National Academy of Sciences (341)

Abstract

RPE65 is an abundant protein in the retinal pigment epithelium. Mutations in RPE65 are associated with inherited retinal dystrophies. Although it is known that RPE65 is critical for regeneration of 11- cis retinol in the visual cycle, the function of RPE65 is elusive. Here we show that recombinant RPE65, when expressed in QBI-293A and COS-1 cells, has robust enzymatic activity of the previous unidentified isomerohydrolase, an enzyme converting all-trans retinyl ester to 11- cis retinol in the visual cycle. The initial rate for the reaction is 2.9 pmol/min per mg of RPE65 expressed in 293A cells. The isomerohydrolase activity of RPE65 requires coexpression of lecithin retinol acyltransferase in the same cell to provide its substrate. This enzymatic activity is linearly dependent on the expression levels of RPE65. This study demonstrates that RPE65 is the long-sought isomerohydrolase and fills a major gap in our understanding of the visual cycle. Identification of the function of RPE65 will contribute to the understanding of the pathogenesis for retinal dystrophies associated with RPE65 mutations.

Bibliography

Moiseyev, G., Chen, Y., Takahashi, Y., Wu, B. X., & Ma, J. (2005). RPE65 is the isomerohydrolase in the retinoid visual cycle. Proceedings of the National Academy of Sciences, 102(35), 12413â12418.

Authors 5

Gennadiy Moiseyev (first)
Ying Chen (additional)
Yusuke Takahashi (additional)
Bill X. Wu (additional)
Jian-xing Ma (additional)

References 32 Referenced 408

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Dates

Type	When
Created	20 years ago (Aug. 22, 2005, 8:34 p.m.)
Deposited	3 years, 4 months ago (April 12, 2022, 2:27 p.m.)
Indexed	1 week, 4 days ago (Aug. 22, 2025, 12:46 a.m.)
Issued	20 years ago (Aug. 22, 2005)
Published	20 years ago (Aug. 22, 2005)
Published Online	20 years ago (Aug. 22, 2005)
Published Print	20 years ago (Aug. 30, 2005)

Funders 0

None

BibTeX

@article{Moiseyev_2005, title={RPE65 is the isomerohydrolase in the retinoid visual cycle}, volume={102}, ISSN={1091-6490}, url={http://dx.doi.org/10.1073/pnas.0503460102}, DOI={10.1073/pnas.0503460102}, number={35}, journal={Proceedings of the National Academy of Sciences}, publisher={Proceedings of the National Academy of Sciences}, author={Moiseyev, Gennadiy and Chen, Ying and Takahashi, Yusuke and Wu, Bill X. and Ma, Jian-xing}, year={2005}, month=aug, pages={12413–12418} }

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